Author: M. Auron, M.D, Department of Hospital Medicine, Cleveland Clinic
Reviewer: V. Dimov, M.D., Department of Hospital Medicine, Cleveland Clinic
A 68 year-old AAF with a history of diabetes mellitus, hypertension, remotely treated breast cancer and sarcoidosis (pulmonary, cutaneous and hepatic) presented with four days of rapidly progressive right frontal and periorbital headache, associated with right ptosis, diplopia and unsteady gait. Negative symptoms for dysarthria, dysphonia, weakness and numbness.
Prednisone 20 mg daily for treatment of sarcoidosis, Metformin 850 mg bid, Pioglitazone 45 mg daily, Metoprolol succinate 100 mg daily, Hydrochlorothiazide 25 mg daily, ASA 81 mg daily, Pravastatin 20 mg daily.
Right ptosis, incomplete elevation, adduction, abduction, anisocoria with right mydriasis with minimal pupillary response to light were present. Her right nasolabial fold was mildly depressed. The remainder of the neurological exam was normal.
What is the most likely diagnosis?
Neurosarcoidosis vs. cerebrovascular accident.
What tests would you order?
- BMP, CBCD, coagulation profile
- ACE levels
- Head computed tomography without contrast
- Brain MRI and MRA with gadolinium
- ACE level 19 U/L (normal)
- Head CT: normal
- Brain MRI with gadolinium: right parasellar mass extending into the cavernous sinus and Meckel’s cave without meningeal enhancement (Fig. 1)
- Brain MRA: anterior and inferior displacement of the right carotid artery (intracavernous portion) (Fig 2)
What further testing would you order?
- CSF looking for findings suggestive of neurosarcoidosis (high protein level, lymphocytosis, normal or low glucose coupled with negative cytology and culture)
- Hormonal profile – as the patient has parasellar mass to rule out affection of pituitary function.
- Cerebrospinal fluid: clear, colorless; 6 WBC, 101 RBC, 2% PMN, 44% Lymphocytes, 41% Monocytes, 13% Macrophages, Glucose 170 mg/dL, Total protein 100 mg/dL.
- Hormonal profile: Normal. T4 7.6 ug/dL, TSH 0.400 uIU/mL, Cortisol, AM 33.7 ug/dL, Free T4 1.2 ng/dL, Prolactin 6.1 ng/mL, FSH 4.4 mIU/mL,
What treatment would you start for this patient?
Stress steroid dose was provided to the patient.
Neurosurgical consultation was requested.
Decompressive surgery was performed with transphenoidal resection of the tumor. There were no significant postoperative complications and the patient was discharged to a rehabilitation facility without requiring immediate hormonal replacement therapy.
The anatomopathologic diagnosis was surprising: Non functioning pituitary adenoma with areas of pituitary apoplexy. (Fig 3)
Fig. 3. The preparation is highly cellular showing a single population of epithelioid cell with eosinophilic cytoplasm and round to oval nuclei with delicate chromatin pattern. Permanent section shows sheets of expanded acini composed of homogeneous polyhedral cells with bland nuclei with “salt and pepper” chromatin pattern. Large areas of ischemic necrosis are noted
Nonfunctioning pituitary adenoma in a patient with systemic sarcoidosis.
What did we learn from this case?
Pathologic diagnosis in the setting of neurologic involvement in patients with multiorgan sarcoidosis remains critical to therapy as potential alternative diagnoses can coexist.
1. Vilar H, Nobre EL, Jorge Z, Filipe JS, Castro JJ. Pituitary macroadenoma associated with pulmonary and cutaneous sarcoidosis - a clinical case. Acta Med Port. 2005;18(4):309-13.
2. 2. Golkowski F, Trofimiuk M, Huszno B, Szybinski Z, Adamek D, Papla B, Stachura J. Nonfunctioning pituitary adenoma and pulmonary sarcoidosis - a case report. Przeg Lek. 2002;59(12):1038-40.